Spinal cord malformations may originate disorders in the innervation of the lower urinary tract. In extreme cases, this involvement leads to a complete disarrange in the crosstalk between the central nervous system and the bladder, sphincters, and pelvic floor. Bladder hyperactivity is a common feature in many of these cases, often resulting in urinary incontinence and loss of bladder compliance and functional capacity in the long term. The treatment of these patients should be highly individualized, with emphasis on managing expectations and priorities. In cases of complete dysfunction of the lower urinary tract, supratrigonal cystectomy with an incontinent urinary diversion is a legitimate option to be discussed with patients and caregivers, as it may offer an improvement in their quality of life. However, the presence of severe constipation, often observed in patients with tetraplegia, increases the likelihood of postoperative ileus and the risks associated with intestinal conduits. In these cases, the construction of cutaneous ureterostomies is a possibility, although often linked to a higher risk of infection and stenosis, which may lead to the need for lifelong ureteral catheterization.

We describe the clinical case of a 19-year-old male patient with Chiari malformation type II, associated with lumbosacral myelomeningocele with tethered spinal cord syndrome and tetraplegia. He presented with a prolonged history of overactive bladder, resulting in urinary incontinence and loss of bladder capacity and compliance. Additionally, he had severe constipation, requiring manual removal of fecalomas. To contain permanent urinary incontinence, the patient had been under chronic bladder catheterization since childhood, with consequent severe traumatic hypospadias and recurrent urinary tract infections (UTI). In the face of an end-stage neurogenic bladder, the possibility of supratrigonal cystectomy and incontinent urinary diversion, namely through ureteroileostomy, was discussed with the patient and caregivers.

The patient underwent open supratrigonal cystectomy. Intraoperatively, a dolichocolon with significant redundancy and dilation of ileum and colon loops was found. Preservation of the entire length of the ureters, including the ureteral orifices, was performed, which were resected with approximately 1cm margin via deep midline cystotomy. The left ureter was transposed under the sigmoid colon to the right side. Cutaneous ureterostomies (with ureteral orifice preservation) were performed in a single stoma in the lower right abdomen, maintaining catheterization with monoJs. The postoperative period was uneventful, with discharge on the 6th day. The monoJ stents were removed on the 14th day post-operatively. In the 6 months of follow-up there was no episode of UTI, serum creatinine is 0.5mg/dL and there was a clear improvement in the patient’s quality of life.

We present the first case of cutaneous ureterostomies with preservation of the ureteral orifices, to our knowledge. As most cystectomies are performed in an oncological context, intravesical manipulation is not possible, nor is the maintenance of the entire length of the ureters, as oncological control is the main objective of the surgery. However, in cases of non-oncological cystectomy, the maintenance of the maximum length of the ureter and even the ureteral orifices may, in theory, decrease the incidence of complications, particularly infections and ureteral stenosis. In fact, preserving the full length of the ureters does not increase the surgical difficulty or time, since it facilitates the subsequent anastomosis to an ileal conduit or the construction of a direct stoma, in addition to dispensing the isolation of the ureters prior to the cystectomy itself. Thus, in cases of non-oncological cystectomies, manipulation of the ureteral mucosa seems unnecessary, with potential benefits particularly in terms of stenosis rate.

Costello AJ, Crowe H, Agarwal D. Supratrigonal cystectomy and ileocystoplasty in management of interstitial cystitis. Aust N Z J Surg. 2000 Jan;70(1):34-8. doi: 10.1046/j.1440-1622.2000.01739.x. PMID: 10696940